Z Gastroenterol 2019; 57(03): 312-316
DOI: 10.1055/a-0829-7050
Kasuistik
© Georg Thieme Verlag KG Stuttgart · New York

Severe ileocecal inflammatory syndrome in adult patients with cystic fibrosis

Ileozökales inflammatorisches Syndrom bei erwachsenen Patienten mit Zystischer Fibrose
Robert Henker
1   University Hospital Leipzig, Division of Gastroenterology, Leipzig, Germany
,
Annett Oltmanns
2   University Hospital Leipzig, Division of Pulmonology, Leipzig, Germany
,
Alexandra Wald
2   University Hospital Leipzig, Division of Pulmonology, Leipzig, Germany
,
Jan Tuennemann
1   University Hospital Leipzig, Division of Gastroenterology, Leipzig, Germany
,
Sabine Opitz
3   University Hospital Leipzig, Institute of Pathology, Leipzig, Germany
,
Albrecht Hoffmeister
1   University Hospital Leipzig, Division of Gastroenterology, Leipzig, Germany
,
Hubert Wirtz
2   University Hospital Leipzig, Division of Pulmonology, Leipzig, Germany
,
Joachim Mössner
1   University Hospital Leipzig, Division of Gastroenterology, Leipzig, Germany
,
Boris Jansen-Winkeln
4   University Hospital Leipzig, Department of Visceral, Transplant, Thoracic and Vascular Surgery, Leipzig, Germany
,
Thomas Karlas
1   University Hospital Leipzig, Division of Gastroenterology, Leipzig, Germany
› Author Affiliations
Further Information

Publication History

28 September 2018

27 December 2018

Publication Date:
12 March 2019 (online)

Abstract

The relevance of gastrointestinal manifestations of cystic fibrosis (CF) is increasing due to an improved life expectancy. We report on 2 adult patients with prior lung transplantation who presented with a severe inflammatory disorder of the ileocecal region. One patient underwent ileocecal resection; the second patient died after emergency surgery for intestinal perforation. Both cases did not show typical signs of CF-related distal intestinal obstruction syndrome or extensive fibrosing colonopathy. However, the clinical and histopathological findings revealed CF-induced inflammatory alterations of the intestinal mucosa. Thus, these cases illustrate a further CF-related bowel disorder, which can be especially relevant in long-term CF survivors.

Zusammenfassung

Aufgrund der gestiegenen Lebenserwartung von Patienten mit Zystischer Fibrose (CF) nimmt die Bedeutung gastrointestinaler Manifestationen der Erkrankung zu. In zwei Fallberichten stellen wir erwachsene, lungentransplantierte CF-Patienten mit ausgeprägten inflammatorischen Veränderungen der Ileozökalregion vor, welche letztlich einer operativen Versorgung zugeführt werden mussten. In beiden Fällen konnten keine eindeutigen Zeichen eines distalen intestinalen Obstruktionssyndroms oder einer zugrundeliegenden fibrosierenden Kolonopathie festgestellt werden. Dennoch sprechen sowohl das klinische Erscheinungsbild als auch die histopathologischen Befunde für das Vorliegen einer CF-induzierten entzündlichen intestinalen Erkrankung. Daher muss von einer bisher nicht ausreichend charakterisierten CF-Manifestation des Intestinums ausgegangen werden, welche besonders für erwachsene CF-Patienten von klinischer Relevanz ist.

 
  • References

  • 1 Kelly T, Buxbaum J. Gastrointestinal manifestations of cystic fibrosis. Dig Dis Sci 2015; 60: 1903-1913
  • 2 Leung DH, Narkewicz MR. Cystic Fibrosis-related cirrhosis. J Cyst Fibros 2017; 16 (Suppl. 02) S50-S61
  • 3 Abraham JM, Taylor CJ. Cystic fibrosis & disorders of the large intestine: DIOS, constipation, and colorectal cancer. J Cyst Fibros 2017; 16 (Suppl. 02) S40-S49
  • 4 Maisonneuve P, Marshall BC, Lowenfels AB. Risk of pancreatic cancer in patients with cystic fibrosis. Gut 2007; 56: 1327-1328
  • 5 MacKenzie T, Gifford AH, Sabadosa KA. et al. Longevity of patients with cystic fibrosis in 2000 to 2010 and beyond: survival analysis of the Cystic Fibrosis Foundation patient registry. Ann Intern Med 2014; 161: 233-241
  • 6 Sabharwal S. Gastrointestinal manifestations of cystic fibrosis. Gastroenterol Hepatol (N Y) 2016; 12: 43-47
  • 7 FitzSimmons SC, Burkhart GA, Borowitz D. et al. High-dose pancreatic-enzyme supplements and fibrosing colonopathy in children with cystic fibrosis. N Engl J Med 1997; 336: 1283-1289
  • 8 Smyth RL, van Velzen D, Smyth AR. et al. Strictures of ascending colon in cystic fibrosis and high-strength pancreatic enzymes. Lancet 1994; 343: 85-86
  • 9 Terheggen G, Dieninghoff D, Rietschel E. et al. Successful non-invasive treatment of stricturing fibrosing colonopathy in an adult patient. Eur J Med Res 2011; 16: 411-414
  • 10 Rovner AJ, Schall JI, Mondick JT. et al. Delayed small bowel transit in children with cystic fibrosis and pancreatic insufficiency. J Pediatr Gastroenterol Nutr 2013; 57: 81-84
  • 11 Werlin SL, Benuri-Silbiger I, Kerem E. et al. Evidence of intestinal inflammation in patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 2010; 51: 304-308
  • 12 Trang T, Chan J, Graham DY. Pancreatic enzyme replacement therapy for pancreatic exocrine insufficiency in the 21(st) century. World J Gastroenterol 2014; 20: 11467-11485
  • 13 Miragoli F, Federici S, Ferrari S. et al. Impact of cystic fibrosis disease on archaea and bacteria composition of gut microbiota. FEMS Microbiol Ecol 2017; 93: fiw230
  • 14 de Simone P, Journé S, Féron P. et al. Right colon perforation in a lung graft patient. Transplantation 2004; 77: 1919-1920
  • 15 Dialer I, Hundt C, Bertele-Harms RM. et al. Sonographic evaluation of bowel wall thickness in patients with cystic fibrosis. J Clin Gastroenterol 2003; 37: 55-60
  • 16 Hadjiliadis D, Khoruts A, Zauber AG. et al. Cystic fibrosis colorectal cancer screening consensus recommendations. Gastroenterology 2018; 154: 736-745
  • 17 Häusler M, Meilicke R, Biesterfeld S. et al. First adult patient with fibrosing colonopathy. Am J Gastroenterol 1998; 93: 1171-1172
  • 18 Mack EH, Brett AS, Brown D. Fibrosing colonopathy in an adult cystic fibrosis patient after discontinuing pancreatic enzyme therapy. South Med J 2004; 97: 901-904
  • 19 Franzen D, Went P, Buhlmann U. Fibrosing colonopathy in absence of pancreatic enzyme supplementation in one adult patient with cystic fibrosis. Indian J Gastroenterol 2008; 27: 133-134